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Adolescent pregnancy remains a health issue worldwide also in developed countries, since it has been associated with adverse maternal and neonatal outcomes. Some data suggest that very young adolescents have higher risk, likely due to immaturity. Therefore, we aimed to assess the influence of maternal age on complications during gestation and labor in pregnant women between 13 and 19 years of age. In particular, we evaluated the possible association between maternal age and obstetric, fetal and labor complications. This is a retrospective, observational and exploratory study conducted at Hospital Universitario La Paz (HULP, Madrid, Spain). The clinical history of 279 women who delivered between 2013 and 2018 was analyzed. Maternal age and the presence of maternal, fetal and labor complications, as well as risk of postpartum depression and breastfeeding intention, were recorded. General regression models were used to analyze the contribution of maternal age on each complication. The percentage of adolescent pregnancies at HULP between 2013 and 2018 was 0.9%. The risk of all the maternal complications analyzed decreased significantly with every year of age of the mother (hyperemesis, lower back pain, anemia, gestational diabetes mellitus, and threat of premature labor and premature rupture of membranes). Every year of maternal age decreased 0.8-fold [0.8; 0.9] the prevalence of fetal complications and also reduced the risk of C-section, postpartum hemorrhage and obstetrical hysterectomy. Furthermore, higher maternal age increased 1.1-fold [1.0; 1.2] the breastfeeding intention. In conclusion, young adolescents are at higher risk of complications during pregnancy and labor.

La enfermedad de Fabry es una entidad clínica infrecuente, que se produce por el acúmulo de trihexosidogalactosilglucosil ceramido debido a un déficit congénito de alfa galactosidasa. La herencia se trasmite de modo recesivo ligada al cromosoma X, siendo las manifestaciones clínicas más severas en varones hemicigóticos. Se caracteriza por angioqueratomas, hipohidrosis, opacificación corneal, alteraciones del cristalino, acroparestesias y lesiones vasculares que pueden afectar a distintos órganos: riñón, corazón y cerebro (1-3).Se han descrito en la literatura varios casos de enfermedad de Fabry coexistiendo con enfermedades del tejido conectivo, en concreto con lupus eritematoso sistémico (4-7) y con un síndrome de Raynaud, eritermalgia y acrodinia (8).Presentamos una asociación no descrita previamente en la que se diagnosticó una artritis reumatoide en una paciente con enfermedad de Fabry. CASO APORTADOMujer de 45 años con antecedentes familiares de padre muerto a los 49 años por insuficiencia renal crónica y con episodios de dolor [0212-7199(2003) (Madrid) 2003; 20: 28-30. RESUMENLa enfermedad de Fabry es una alteración congénita poco frecuente, del metabolismo de los glucoesfingolípidos, en la cual existe un déficit de la enzima alfa galactosidasa A. Se trasmite de modo recesivo ligada al X por lo que las manifestaciones clínicas son más severas en varones hemicigóticos. El diagnóstico se lleva a cabo mediante la determinación de los niveles del enzima alfa-galactosidasa.En la literatura se han descrito varios casos de enfermedad de Fabry asociada a enfermedades del tejido conectivo, pero no se ha encontrado ningún caso coexistiendo con artritis reumatoide.Presentamos un caso de una paciente con enfermedad de Fabry que, posteriormente se diagnosticó de artritis reumatoide. El diagnóstico de sospecha fue fundamental ya que el carácter multisistémico de las manifestaciones clínicas de ambas enfermedades hace que sea difícil no atribuir nuevos signos a la enfermedad de Fabry.El acúmulo de lípidos provoca un estímulo antigénico prolongado que es capaz de formar inmunocomplejos. Esta es la hipótesis patogénica por la que creemos que la enfermedad de Fabry se asocia a enfermedades autoinmunes.

Introduction: Critically ill children in the pediatric intensive care unit (PICU) are at high risk for developing nutritional deficiencies and undernutrition is known to be a risk factor for morbidity and mortality. Malnutrition represents a continuous spectrum ranging from marginal nutrient status to severe metabolic and functional alterations and this in turn, affects clinical outcome. Objectives: The aim of the study was to assess nutritional status of critically ill children admitted to the PICU and its association to clinical outcomes. Methods: Critically ill children age 6 months to 18 years were prospectively enrolled on PICU admission. Nutritional status was assessed by weight for age (WFA: underweight), weight for height (WFH: wasting), height for age (HFA: stunting) z-scores and mid upper arm circumference (MUAC: wasting) according to the WHO. (1,2) Malnutrition was defined as mild, moderate, and severe if z-scores were > −1, > − 2, and > −3, respectively. Hospital and PICU length of stay (LOS), duration of mechanical ventilation (MV), and risk of mortality (ROM) by the Pediatric Index of Mortality 2 (PIM2) were obtained. Sensitivity and specificity of the MUAC to identify children with wasting (WFH) were calculated. Results: Two hundred and fifty children (136 males), aged 81 months (23-167; median (25-75 th IQR)), were prospectively included in the study. The hospital LOS was 8 (4-16) days; PICU LOS: 2 (1-4) days; duration of MV, 0 (0-1.5) days;

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